|Year : 2016 | Volume
| Issue : 1 | Page : 85-88
Takayasu arteritis presenting as bowel gangrene: An unusual initial presentation
Richa Dewan, Subramanian Anuradha, Prayas Sethi, Pranav Ish
Department of Medicine, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
|Date of Web Publication||2-Jun-2016|
4/56 Mehrauli, New Delhi - 110 030
Source of Support: None, Conflict of Interest: None
A 24-year-old lady presented to the emergency department with severe generalized abdominal pain and hematochezia of 2 days. Examination revealed absent bilateral radial, brachial, and carotid pulses. Rest of the peripheral pulses were normal. A bruit was heard over bilateral carotid, subclavian, and renal arteries. X-ray abdomen showed dilated bowel loops with multiple air fluid levels. Doppler study revealed intimal thickening in bilateral subclavian and common carotid arteries along with decreased flow. Computed tomography (CT) angiography was done urgently that showed critical narrowing of the bilateral subclavian, common carotid, coeliac trunk, superior mesenteric arteries, inferior mesenteric arteries, and bilateral renal arteries. We present a case of mesenteric ischemia in a patient of Takayasu arteritis (TA) leading to bowel gangrene involving entire small and large bowel and a fulminant outcome, which is an uncommon initial presentation of TA.
Keywords: Mesenteric ischemia, takayasu arteritis (TA), bowel gangrene
|How to cite this article:|
Dewan R, Anuradha S, Sethi P, Ish P. Takayasu arteritis presenting as bowel gangrene: An unusual initial presentation. Arch Med Health Sci 2016;4:85-8
|How to cite this URL:|
Dewan R, Anuradha S, Sethi P, Ish P. Takayasu arteritis presenting as bowel gangrene: An unusual initial presentation. Arch Med Health Sci [serial online] 2016 [cited 2020 Feb 29];4:85-8. Available from: http://www.amhsjournal.org/text.asp?2016/4/1/85/183340
| Introduction|| |
Takayasu arteritis (TA) is a chronic vasculitis that usually affects young women and has higher prevalence in Asian subcontinent. It most commonly affects the aorta and its major branches. Although there is considerable variability in disease expression, the initial vascular lesions frequently occur in the left middle or proximal subclavian artery. The common consequences of these lesions are neurological, renal, abdominal, and cardiac insults and claudication of limbs. Mesenteric artery involvement is a comparatively rare initial presentation of TA. We present a case of mesenteric ischemia in a patient of TA leading to bowel gangrene involving entire small and large bowel and a fulminant outcome, which is an uncommon initial presentation of TA and its exact occurrence is not well defined.
| Case Report|| |
A 24-year-old lady presented to the emergency department with severe generalized abdominal pain and hematochezia of 2 days. The patient had a history of dull aching and intermittent abdominal pain for the past 4 months that increased with food intake and was relieved on its own a few hours after meal. However, over the past 2 days the patient had developed constant pain of severe intensity that was also associated with episodes of bilious vomiting. She also had hematochezia since past 2 days.
Examination revealed a pale young female with absent bilateral radial, brachial, and carotid pulses. Rest of the peripheral pulses was normal. A bruit was heard over bilateral carotid, subclavian, and renal arteries. Blood pressure in the upper limb was not recordable while in lower limb it was 200/100 mmHg. Abdominal examination revealed diffuse tenderness with guarding. Bowel sounds were diminished. Rest of the systemic examination was unremarkable.
Investigations revealed hemoglobin level of 6.8 g/dL, total leukocyte count (TLC) of 15,500/µL, and platelet count of 2.6 lac/µL with erythrocyte sedimentation rate (ESR) of 81 mm/h. Serum creatinine level was 1.3 mg%. Rest of the routine blood investigations was normal. X-ray abdomen showed dilated bowel loops with multiple air fluid levels. Doppler study revealed intimal thickening in bilateral subclavian and common carotid arteries along with decreased flow. There was normal flow in lower limbs arteries. CT angiography was done urgently [Figure 1], [Figure 2], [Figure 3], [Figure 4] that showed critical narrowing of the bilateral subclavian, common carotid, coeliac trunk, superior mesenteric arteries, inferior mesenteric arteries, and bilateral renal arteries.
|Figure 1: Dilated small bowel loops with air fluid level (white arrow) with decreased mesenteric vascularity. Also seen is narrowed inferior mesenteric artery arising from anterior wall of aorta (black arrow)|
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|Figure 2: Narrowed coeliac, superior mesenteric, and inferior mesenteric arteries seen arising from aorta. Collateral vessels from bilateral iliac vessels, anastomosing with mesenteric vasculature in response to chronic mesenteric ischemia|
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|Figure 3: MIP image reveals narrowed bilateral renal arteries (black arrow) with decreased intrarenal vascularity with feeding collaterals from bilateral iliac vessels|
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|Figure 4: Markedly narrowed celiac axis arising from aorta (as marked by arrow). Also seen in this image are collateral vessels in the anterior wall of abdomen|
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The patient was treated conservatively with antibiotics, intravenous fluids, and Ryle's tube aspiration. Blood was transfused. In view of mesenteric ischemia, the patient was planned for revascularization procedure. The patient deteriorated on the second day of hospital stay with worsening of symptoms and developed abdominal rigidity. An emergency laparotomy was done that revealed gangrene extending from the proximal stomach to the rectum and resection of the gangrenous part was done extending from the whole of duodenum and small and large intestine upto the rectum resected. Proximal gastrostomy was created. The patient however succumbed to her illness postoperatively.
| Discussion|| |
Chronic mesenteric ischemia (CMI) is a morbid disease that results from progressive stenotic disease of the mesenteric vessels. Atherosclerosis is the most common cause, with fibromuscular dysplasia, polyarteritis nodosa, and TA representing rare causes.
TA is a chronic vasculitis of unknown etiology. It mainly affects young women in the second and third decades of life. It has a worldwide distribution, with the greatest prevalence among Asians.
It primarily affects the aorta and its branches. Systemic symptoms, including fatigue, weight loss, and low-grade fever, are common in the early phase of disease. Vascular symptoms are rare at presentation, but with progression of disease, evidence of vascular involvement, and insufficiency becomes clinically apparent due to dilation, narrowing, or occlusion of the proximal or distal branches of the aorta. Aorta is involved along the entire length, with subclavian and common carotid arteries being most commonly affected. Stenotic lesions are predominant (>90%), whereas aneurysms are reported in approximately 25%.
Subclavian artery involvement is common, and a stenotic lesion proximal to the origin of the vertebral artery can lead to neurologic symptoms. Other symptoms include arthralgias; myalgia; skin lesions; symptoms related to pulmonary, carotid, vertebral, and coronary artery involvement; abdominal pain; and nausea, vomiting, and diarrhea that may result from mesenteric artery ischemia, which is rare in TA.
In a case series of 33 patients with descending thoracic or thoracoabdominal aortic aneurysm in association with TA, only 7 patients had thoracic or abdominal pain as initial presentation. Chronic and refractory abdominal pain can be a presenting feature of mesenteric ischemia in TA. Mesenteric infarction, on the other hand, is rarer. Mehta et al . reported a case of a 40-year-old man with TA involving the abdominal aorta, who presented with CMI. The diagnosis was made by aortogram, which showed tight superior mesenteric artery (SMA) stenosis. The patient underwent successful balloon angioplasty of the SMA following corticosteroid treatment. However, cases have been reported with mesenteric infarction, often leading to a fulminant outcome. Most cases have reported small bowel infarction and gangrene because of superior mesenteric and celiac axis involvement.,,, A case of isolated ischemic colitis has also been reported due to involvement of inferior mesenteric artery.
Surgical treatment involves revascularization of the stenotic or occluded mesenteric vessels. Inflow may be from the supraceliac aorta (antegrade reconstruction) or from the infrarenal aorta or the common iliac artery (retrograde reconstruction). Outflow may be to the celiac or more commonly to the SMA only (single-vessel repair) or to a combination of celiac artery (CA), SMA, and rarely inferior mesenteric artery (IMA) (multivessel repair). Endovascular therapy also has been used to treat CMI. Although no prospective randomized control data are available, studies indicate that in the short term, percutaneous angioplasty with or without stenting is effective  although mid-term and long-term data are not available. The advantage of endovascular therapy is its minimally invasive nature. Its disadvantages are the need for reintervention and inferior durability.
Thus, a high index of suspicion must be kept to look for mesenteric ischemia associated with bowel gangrene in patients presenting with acute abdomen and having asymmetrical pulses, as early diagnosis and timely intervention are key to prevent morbidity and mortality.
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Conflicts of interest
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]